Major Review

SURVEY OF OPHTHALMOLOGY VOLUME 52 NUMBER 5 SEPTEMBER–OCTOBER 2007

MAJOR REVIEW

Diagnosis and Management of Enophthalmos Mehrad Hamedani, MD,1 Jean-Antoine C. Pournaras, MD,1 and David Goldblum, MD2

1Jules Gonin Eye Hospital—University of Lausanne, Lausanne, Switzerland; and 2Universita¨ts-Augenklinik, University of Basel, Basel, Switzerland

Abstract. Enophthalmos is a relatively frequent and misdiagnosed clinical sign in orbital diseases. The knowledge of the different etiologies of enophthalmos and its adequate management are important, because in some cases, it could be the ﬁrst sign revealing a life-threatening disease. This article provides a comprehensive review of the pathophysiology, evaluation, and management of enophthalmos. The main etiologies, such as trauma, chronic maxillary atelectasis (silent sinus syndrome), breast cancer metastasis, and orbital varix, will be discussed. Its objective is to enable the reader to recognize, assess, and treat the spectrum of disorders causing enophthalmos. (Surv Ophthalmol 52:457--473, 2007. Ó 2007 Elsevier Inc. All rights reserved.)

Key words. breast cancer metastasis enophthalmos fat atrophy orbital fracture pseudoenophthalmos scleroderma silent sinus syndrome trauma varix

I. Diagnosis of Enophthalmos 2. Clinical Examinaton A. DEFINITION Enophthalmos is often obvious during the in- Enophthalmos is a posterior displacement of the spection of a patient’s face. The diagnosis is eyeball within the orbit in an antero-posterior plane simpliﬁed in cases of unilaterality or major asym- due to several etiologies.33 The volume of the globe is metry. Indirect clinical signs contribute to the normal. In case of unilaterality, a difference of more diagnosis of enophthalmos and include deep than 2 mm between the two eyes can be considered superior sulcus, narrowing of the palpebral ﬁssure diagnostic. It is the opposite of exophthalmos (pseudoptosis), and lagophthalmos. (proptosis) where the globe is pushed forward. The position of the globe in the orbit has a high variability due to age, sex, and ethnic background. The best position for the clinical recognition of enophthalmos is asking the patient to look up with B. CLINICAL PRESENTATION the head tilted back, and the observer being in front of the patient (Fig. 1). Objective and quantitative 1. Symptoms measurement can be achieved by Hertel exophthal- Subjective complaints depend strongly on the mometry. In case of orbital fractures with displace- etiology and severity of enophthalmos. The most ment of the lateral orbital rim, other devices using common disturbances are facial asymmetry and a frontal support are necessary (e.g., Naugle exoph- double vision. Sometimes, the patient may consider thalmometer). Concomitant vertical misalignement the disorder as a ptosis or contralateral proptosis. (hypoglobus) is often present.

disease. Due to the reduced volume, the eye will appear sunken in to the orbit and the lids will seem ptotic without actual axial displacement of the globe in relation to its surrounding structures (Fig. 2A).3

b. Microphthalmos, Microcornea Microphthalmos is defined as a congenitally small eye with reduction of the volume of the globe in the absence of other ocular anomalies.42,49 On the basis of the small corneal diameters the diagnosis is obvious and seldom missed, even in young infants. As described for phthisis bulbi, the volume reduction Fig. 1. Left enophthalmos. of the globe or anterior segment will make the eye appear enophthalmic. Microphthalmos could be C. RADIOLOGICAL IMAGING part of hemifacial microsomia (Fig. 2B). Radiological investigations, computed tomography (CT) scan and magnetic resonance imaging c. Refractive-Anisometropia (MRI), confirm and also quantify enophthalmos. In case of significant anisometropia the shorter Axial sections in the neuro-ocular plane provide eye may lead to the wrong impression of being reproducible measurements and can be used for enophthalmic. It should be noted that the general 22,170 follow-up comparison. Coronal and sagittal rule that 3 diopters translated into 1 mm of sections are equally important for the analysis of biometric axial length may sometimes be mislead- the surrounding tissues and sinuses. The CT scan ing, given the widespread use of refractive surgery. serves as the reference for the analysis of the bony structures (orbital container), whereas the MRI is 2. Altered Lid Position more relevant for the observation of the globe surrounding soft tissues (orbital content). a. Horner Syndrome The syndrome named after Johann Friedrich D. PSEUDOENOPHTHALMOS Horner76 has generally been described with miosis, The definition of true enophthalmos has been ptosis, and enophthalmos, as well as anhidrosis. described in previous sections; therefore, it is A lesion at any point along the oculosympathetic pathway will result in this syndrome with symptoms important to distinguish disorders that may initially 86 appear as enophthalmos, due to lid malpositions, on the same side and anisocoria. Anisocoria is globe size anomalies, or structural deviations (Table more apparent in dim illumination, and the 1), but are not associated with an actual axial affected pupil shows dilation lag. Light and near displacement of the globe. pupillary reactions are intact. The eyelid is ptotic because of paresis of the sympathetically innervated Mu¨ller’s muscle. There seems to be apparent 1. Globe enophthalmos (pseudoenophthalmos) due to the a. Phthisis Bulbi ptosis and because the lower eyelid may be elevated; Phthisis bulbi is defined as a shrinking of the exophthalmometry readings, however, are generally 108,126,175 globe often following injury, surgery, infection, or equal (Fig. 2C).

b. Ptosis TABLE 1 Ptosis is deﬁned as a drooping of one or both Etiologies of Pseudoenophthalmos eyelids. It may be complete or incomplete, varying Globe Phthisis bulbi in degree of severity. As described in the Horner Microphthalmos, microcornea syndrome section, a blepharoptosis can lead to the Refractive-Anisometropia impression of an enophthalmos (Fig. 2D).8 Altered lid position Horner’s syndrome Ptosis Contralateral lid retraction 3. Structural Lesions Structural lesions Post Enucleation Socket Syndrome (PESS)/Anophthalmic socket a. Post-Enucleation Socket Syndrome (PESS) Contralateral exophthalmos Marked pseudoenophthalmos frequently occurs Facial/Bony asymmetry after enucleation with or without the use of intra- DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 459

Fig. 2. A: Left pseudoenophthalmos: Phthisis Bulbi. B: Right pseudoenophthalmos: Microphthalmos. C: Right pseudoenophthalmos: Horner’s syndrome. D: Right pseudoenophthalmos: Congenital Ptosis. orbital implants (Fig. 3). It is often associated with condition on the contralateral side. Therefore, any a superior sulcus syndrome, which is another difference in the position of the eyes has to be common ﬁnding in the anophthalmic socket. The carefully evaluated for being pseudoexophthalmic or causes are reduction of orbital content, and pro- pseudoenophthalmic in respect to its side. gressive relaxation of the lower eyelid leading to downward passage of the prosthesis, with associated c. Facial Asymmetry ectropion development and decreased volume of the Any bony malformation in the skull resulting in orbit. The main theory (based on clinical impression) a facial asymmetry can lead to the impression of an of orbital fat atrophy due to metabolic or circulatory enophthalmos due to the asymmetry of the face with alterations was never proven by clinical or experi- 97,98 anterior or posterior displacement of the whole orbit. mental studies. To prevent PESS, careful evalua- The globe, however, might well be in a physiological tion of an adequate-sized implant and correct position in regard to the orbital surroundings. placement during surgery are important. Neverthe- less, if the syndrome becomes apparent, relaxation of the lower eyelid can be corrected with surgical repair II. Pathophysiology of Enophthalmos and if necessary support using, for example, autogenous fascia lata.132 Decreased volume of the orbital Three main mechanisms are proposed in the content can be corrected also with materials such as genesis of enophthalmos: enlargement of the autogenous fat79 or dermis-fat,69,157,162 autogenous orbital container, reduction of the orbital content, 143 cartilage,36 autogenous bone, sclera and liquid and contraction of the orbital content (Table 2). collagen,160 silicone,123,155,167 glass beads,161 or porous polyethylene,18,66,147 using several surgical ap- A. ENLARGEMENT OF THE ORBITAL CONTAINER proaches and techniques (intraorbital, subperiostal). Enlargement of the orbital container seems to be the most frequent cause of enophthalmos. Different b. Contralateral Proptosis/Exophthalmos mechanisms may modify the orbital walls and hence Exophthalmos is deﬁned as a forward displace- increase the orbital volume. It could be a defect of ment of the normal globe in relation to its bony orbit. the orbital wall(s) or an external displacement of This may lead to the impression of an enophthalmic these walls. Orbital fractures, chronic maxillary 460 Surv Ophthalmol 52 (5) September--October 2007 HAMEDANI ET AL

Fig. 3. A: Left pseudoenophthalmos: Post Enucleation Socket Syndrome (PESS). B: CT-Scan Sagittal view showing the posterior and inferior displacement of the implant. C: CT-Scan Sagittal view showing surgical correction by subperiostal orbital grafts. D: Postoperative result after six months. atelectasis, and agenesis of sphenoid wing in responsible for enophthalmos. Fat atrophy can be Recklinghausen disease are the main diagnoses to age-related, or secondary to orbital varices and consider. radiotherapy. Lipodystrophy may also be part of systemic disease or secondary to medical treatment. Scleroderma and hemifacial atrophy are two rare B. REDUCTION OF THE ORBITAL CONTENT causes of orbital atrophy. Among the orbital contents, fat and muscle make up most of the volume. Vessels and nerves do not C. CONTRACTION OF THE ORBITAL CONTENT use enough space that their shrinking could be Some orbital diseases may induce a posterior displacement of the eyeball by developing an intraconal cellular infiltrate with the potential for TABLE 2 contraction. Alternatively, fibrosis and scar forma- Pathophysiology of Enophthalmos tion may lead to contraction with consecutive retraction of the globe. Orbital metastases, particu- Enlargement of orbital Trauma container Chronic maxillary atelectasis larly breast cancer and post-radiotherapy scarring Agenesis of sphenoid wing represent the main etiologies. Congenital fibrosis 64,65,71 Orbital varix should be excluded. Reduction of orbital Age-related fat atrophy content Orbital varix Radiotherapy Lipodystrophy III. Etiologies of Enophthalmos and Linear scleroderma Management Hemifacial atrophy Trauma/Surgery A. POST-TRAUMATIC ENOPHTHALMOS Contraction of orbital Breast cancer metastasis The most frequent cause of enophthalmos is the content Trauma/Fibrosis fracture of the orbital floor. The enlargement of the DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 461 orbital container may also be secondary to medial pupillary dysfunction, epistaxis, and dysesthesia in orbital wall fracture, which is very often misdiagnosed the V2 distribution are often seen. Larger fractures at the early stage of trauma.40 Lateral wall and orbital result in smaller ocular motility dysfunction, due to roof fractures are less frequent and seldom associated the fact that the muscle is not strangulated. Eyeball with enophthalmos. The enlargement of the orbit trauma and other facial fractures have to be ruled towards the periorbital sinuses is explained by two out by careful examination. main theories. The hydraulic theory postulates that Enophthalmos may be missed during the early an increased hydraulic force in the orbit caused by stages of trauma, and may only be detected weeks or posterior eyeball displacement, suddenly increases months after the initial trauma. In these cases, the intraorbital pressure leading to the rupture of the motility disorders are often permanent due to the medial and/or inferior wall. The buckling theory fibrosis. The early CT scan may predict the risk of involves a direct trauma to the inferior orbital rim late enophthalmos according to the extent of the causing mainly the displacement of the floor along fracture.183 the infraorbital channel. The traumatic conditions The amount of enophthalmos is assessed with include sports trauma, vehicular injuries, fighting, Hertel exophthalmometry. If the fracture extends to and, rarely, orbital or endonasal surgeries. the lateral orbital rim, the Naugle exophthalmom- Enophthalmos could be present immediately eter may be useful.151 The Hess-Lancaster test after trauma, or appear later after the reduction of confirms the diagnosis of oculomotor disorders orbital hemorrhage and edema. The early detect- and facilitates the follow-up of the diplopia. able enophthalmos is associated with severe orbital Orbital CT scan (Fig. 4) with axial, coronal, and contusion. In these cases, periorbital hematoma, sagittal sections provides the necessary information edema, subcutaneous emphysema, orbital pain, about the location and the extent of the fracture(s), diplopia, lacrimal system injuries, oculomotor and the involvement of soft tissues (extraocular muscles,

Fig. 4. A: CT scan, axial section, left medial wall fracture. B: CT scan, coronal section, left floor fracture. C: CT scan, sagittal section, orbital floor fracture. 462 Surv Ophthalmol 52 (5) September--October 2007 HAMEDANI ET AL orbital fat),9,67,134,135 and the risk of late enoph- necessary to restore the appropriate orbital shape thalmos.46,47,118,153 Orbital volumetric analysis and and volume before the implant placement. digital reconstructions may be useful for the The surgically most challenging patients are those improvement of the predictive diagnosis of late with the sequellar forms of orbital fractures with enophthalmos and the quality of surgical recon- restrictive fibrosis of the periorbit and the oculo- struction.24,25,41,48 motor muscles. The main risk is worsening of the Standard radiographies should be avoided and diplopia. MRI does not provide any further information for Controversy persists about the choice of the the diagnosis. MRI may contribute to the diagnosis material used for the fracture repair, which of muscular fibrosis, however, in cases of persistent could be autologous (bone or carti- diplopia after fracture reconstruction or muscular lage)27,28,95,102,104,158,184 or synthetic (resorbable restriction in enophthalmic sequelar forms. or not).12,32,48,55,56,66,75,85,87,94,109,122,125,130,147,152,178 The cosmetic demand with or without diplopia is The final surgical result depends mainly on the the main indication for surgery in enophthalmic quality of the three-dimensional reconstruction of patients. As the enophthalmos appears in cases of the orbit (shape and volume) and less on the choice larger fractures, there is rarely muscular suffer- of the orbital implant. However, this material ing.The optimal timing for surgery is 1--2 week(s) should be easy to model and well tolerated. following the trauma. This period allows for the Sometimes the correction of enophthalmos is not resorption of orbital edema and hemorrhage. satisfactory mainly because of undercorrection. The correction of enophthalmos is based on two Different explanations have to be considered: main steps: first, reintegration of the herniated misdiagnosis of the medial wall fracture, lack of orbital content after a careful dissection by appro- material used for the reconstruction or its re- priate approach, and, second, reconstruction of the sorption, or the absence of reconstruction of the orbital shape and volume. orbital floor convexity behind the equator of the The transconjunctival approach14,119 is used in eyeball. small fractures of the orbital floor, whereas a coronal Besides enlargement of the orbital container, two incision helps for complex orbitozygomatic frac- other mechanisms of enophthalmos may play a role tures especially at late stage. In case of orbital floor in traumatic conditions: fat atrophy and contraction fracture, the subciliary incision 2 mm below the lid of the orbital content by fibrosis. margin, or a palpebral incision in the lid crease, provide a good exposure. The transconjunctival approach with lateral cantholysis permits the same B. CHRONIC MAXILLARY ATELECTASIS (SILENT exposure with limited cutaneous scar. The trans- SINUS SYNDROME) caruncular approach13,53 is often useful for the Enophthalmos due to chronic maxillary atelecta- management of the medial wall fracture with sis is very often misdiagnosed. The pathophysiology excellent exposure, without any cutaneous scar. is unknown but it seems that the lateralization of the In orbitozygomatic fractures, different approaches middle turbinate could be the origin of the sinus can be combined: subciliary, palpebral, and trans- disease. In fact, this malposition is almost always vestibular incisions.96 present and could be responsible for negative The endoscopic-assisted transconjunctival ap- pressure in the maxillary sinus and the subsequent proach for medial wall fracture provides improved downward displacement of the orbital floor with visualization of the fracture site, facilitating bony enlargement of the bony orbit and concurrent reduction and the placement of implant.11,30,121 enophthalmos.17,20,21,38,57,72,73,78,81,89,90,99,117,129,142-- The endoscopic transnasal approach has been 146,164,165,176 described also for the treatment of medial orbital The patient may present with a history of sinusitis. wall fractures,105 and the endoscopic-assisted trans- Enophthalmos is progressive over years without any antral approach has been used to repair orbital floor sign of inflammation (Figs. 5A and 5B).165 Vertical fractures.31 diplopia may occur during the evolution secondary Forced-duction testing at the beginning of surgery to the eyeball displacement.43,180,185 provides information about muscular involvement CT scan or MRI will confirm enophthalmos and and has to be repeated at the end of surgery to enlargement of the orbit with downward displace- confirm the release of herniated muscle. ment of the floor and reduction of the sinus cavity. The size and the location of the bony defect will The maxillary sinus mucosa may be thickened. guide the choice and shape of the orbital implant. If There is no history or sign of orbital fracture and surgery is performed late after trauma, especially in the orbital content is normal (Figs. 5C-- orbitozygomatic fractures, osteotomies may be E).20,44,51,81,92,99,103,129,138,164,182 DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 463

Fig. 5. A, B: Left enophthalmos and vertical dystopia. C: CT scan, coronal section, ‘‘implosion’’ of the right maxillary sinus and lateral displacement of the middle turbinate. D: MRI, axial section, right maxillary sinus atelectasis and thickening of the mucosa. E: MRI, Coronal section, Right orbital enlargement and downward displacement of the ﬂoor.

Given the pathophysiological hypothesis, an early correction of enophthalmos, and reaction against middle meatal antrostomy could improve the synthetic orbital implants. aeration of the sinus and possibly reverse the course of the disease.6,17,19,26,70,171 Later, diplopia and C. RECKLINGHAUSEN DISEASE cosmetic demand are the main indications for The absence of the sphenoid wing in some cases surgery.37,82 Subperiosteal orbital ﬂoor grafting is of Recklinghausen disease explains the pulsating a reconstructive option as a secondary procedure. enophthalmos,127 or proptosis (Fig. 6). There is Many surgeons recommend simultaneous sinus a communication between the brain and the orbital drainage and orbital reconstruction, unless marked content.50,61,107,150 Treatment is based on the in- sinus infection is present. terposition of grafts by a neurosurgical approach. The main complications are persistence or de- Autologous bone grafts may be resorbed and terioration of diplopia, undercorrection and over- biomaterials are preferable.163 464 Surv Ophthalmol 52 (5) September--October 2007 HAMEDANI ET AL

Orbital varix could be complicated by hemorrhage or thrombosis. Hemorrhage is responsible for sudden pain and proptosis, oculomotor limitation, and sometimes visual loss. CT scan or MRI shows a diffuse intraconal extravasation of blood. Emergency drainage could be indicated according to the functional signs. The prognosis is very often benign. A particular clinical presentation may be pointed out and called the blocking syndrome. The patient presents with a sudden painful unilateral proptosis. CT scan or MRI shows an oblong limited intraconal process (Fig. 7G). There is a spontaneous clinical and radiological regression. This syndrome could be Fig. 6. CT scan, axial section, absence of the right explained by the filling of the varix followed by sphenoid wing, Recklinghausen disease. outflow limitation. There is no change in the varix, and the Vasalva maneuver persists after the resolu- D. ORBITAL VARIX tion of proptosis. Vascular lesions (lymphangioma, cavernous angi- The treatment of orbital varix is very often oma, capillary angioma) in the orbit are more often conservative and surgical resection is indicated responsible for proptosis rather than enophthal- mainly in cases of anterior thrombosed lesions. mos.68 In some cases, intraoperative direct venography Varix represents a venous anomaly, mainly occur- and embolization by cyanoacrylate glue may greatly ring in the superior ophthalmic vein (SOV), and facilitate the excision of the venous malformation.100 rarely in the inferior vein or both veins. When the dilated vein is empty, enophthalmos appears, and when the venous pressure is high and the varix is E. BREAST CANCER METASTASIS filled, the globe is pushed forward and is proptotic Different cancers (breast, stomach, lung, pros- (Valsalva maneuver; Figs. 7A and 7B). Sometimes tate) may be responsible for enophthalmos by there are also venous malformations of the eyelids contraction of orbital tissues.4,5,29,39,45,59,74,101,110, and the episclera guiding the physician towards this 140,149,156,166,172,179 Breast cancer metastasis should etiology and the diagnostic Valsalva maneuver. The be considered in the initial differential diagnosis for enophthalmos is explained by orbital fat atrophy appropriate-aged females with non-traumatic and sometimes enlargement of orbital space.63 enophthalmos (Figs. 8A and 8B). Enophthalmos Some activities may participate in the develop- due to orbital metastasis could be the first manifes- ment of these venous abnormalities: sports, yoga, tation of the cancer.74,115 Early diagnosis may and playing music instruments. improve the final outcome and prognosis. Patients suffer from intermittent orbital pain or The metastatic retrobulbar infiltrate seems to positional proptosis. The Valsalva maneuver con- have a potential for contraction explaining the firms the venous participation and guides further eyeball displacement and oculomotor disorders.101 investigations. In fact, MRI and CT scan could be The course of the disease is very often insidious normal on decubitus positioning, and the orbital and can be subacute or more often chronic. The varix visible only on procubitus positioning (Figs. enophthalmos appears progressively. The inflamma- 7C--F). Furthermore, spiral CT during Valsalva tory signs may be mild or absent. Eye movements are maneuver using a single breath hold technique also progressively restricted secondary to the evolu- could lead to the diagnosis of this venous anomaly, tion of intraconal infiltration, and diplopia is even in patients who are asymptomatic.148 a frequent motivation for consultation at this stage. Ultrasound-Doppler examination shows a low The vision is unaltered. The disease may be flow, compatible with venous lesions. During ultra- unilateral or bilateral. sound, the Valsalva maneuver is often useful to show MRI is highly superior to CT scan in this the variability of the lesion. pathology. It shows the intraconal infiltrate < Fig. 7. A: Left enophthalmos with vascular anomalies of the left lids. B: Positive Valsalva maneuver. C: MRI, axial section, left intraconal vascular lesion, patient on decubitus. D: MRI, axial section, left intraconal vascular lesion, patient on procubitus. E: MRI, coronal section, left intraconal vascular lesion, patient on decubitus. F: MRI, coronal section, left intraconal vascular lesion, patient on procubitus. G: CT scan, axial section, ‘‘Blocking syndrome.’’ DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 465 466 Surv Ophthalmol 52 (5) September--October 2007 HAMEDANI ET AL

Fig. 8. A, B: Left enophthalmos. C: Mammogram: calcifications, breast cancer. D: MRI, axial section, T1, left intraconal infiltration. E: MRI, axial section, T2, left intraconal infiltration. F: Microscopic view of the infiltration of orbital tissues by malignant cells showing keratin expression. behind the posterior pole of the eye with enoph- by orbital intraconal biopsy (Fig. 8F). The presence thalmos (Figs. 8Dand8E). There is no orbital wall of estrogen and progesterone receptors, identified fracture. by fluorescent histochemical techniques, may guide Further investigations concern mainly gynecolog- the medical treatment.139 Orbital biopsy should be ical status: breast examination, mammogram discussed with oncologists in case of negative (Fig. 8C), breast ultrasound, and particularly breast systemic investigations. cancer circulating markers (i.e., CA 15-3, CEA), Surgery may be difficult because of the topogra- which may be the only sign of generalized cancer at phy of the lesions and the visual risk. Different this stage. A breast biopsy should be considered in approaches are possible, such as a conjunctival case of clinical or radiological mass. The diagnosis approach with disinsertion of the medial or lateral of breast cancer metastasis to the orbit is confirmed rectus muscle, or lateral osteotomy. DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 467

(Figs. 9aand9b). The skin is involved first and appears indurated. Ophthalmic manifestations may include the mentioned atrophy in the orbit and periocular region, sclerosis or inflammation of the eyelids, orbit, or globe.23,136,154,168,169,177 Serologic abnormalities may include anti-nuclear antibodies, anti-single-stranded DNA antibodies, and rheuma- toid factor. Eosinophilia may be present and may correlate with disease activity. A polyclonal IgG and IgM hypergammaglobulinemia may also be present and is found more often with severe cases and with clinical progression.173 According to histopathol- ogy, there are two phases: an early inflammatory phase with coarsened collagen bundles in the reticular dermis and perivascular lymphocytic in- filtrates, and a second late sclerotic phase in which the collagen bundles become hyalinized, replacing muscle and subcutaneous fat. Therapeutic management options have included topical, intralesional, or systemic steroids; vitamin E; vitamin D3; phenytoin; retinoids; penicillin; griseofulvin; interferon-(x);128 D-penicillamine; antimalarials; colchicines; antiplatelet therapy; ultraviolet A phototherapy with or without psoralens; and surgery.80 One case of enophthalmos due to systemic scleroderma has also been described in the literature.93 In contrast to linear scleroderma, Parry-Romberg syndrome (facial hemiatrophy) is characterized by a disappearance of fat in the dermal and subcutaneous tissues on one side of the face. It occurs Fig. 9. A, B: Left enophthalmos: scleroderma. mainly in females, usually within the first two decades of life, and is slowly progressive.124 The affected side of the face is bony, and the skin is thin, The treatment of the generalized cancer is wrinkled, and darkened or brown in its advanced multidisciplinary, including oncologists and gyne- form. The facial hair may turn white and fall out, cologists, and is always based on systemic drugs and the sebaceous glands become atrophic. Muscles (chemotherapy, anti-hormones). The indication for and bones are usually not involved. Sometimes the orbital biopsy should be discussed, and orbital atrophy becomes bilateral and involvement of the radiotherapy may be considered as an adjuvant ipsilateral upper extremity and half of the body has treatment. been described. The disorder may be associated with neurological features.113 Other ocular involvement besides enophthalmos are refractive changes,88 F. LINEAR SCLERODERMA/PARRY-ROMBERG ptosis, restrictive and paralytic strabismus, colobo- SYNDROME (HEMIFACIAL ATROPHY) ma, heterochromia, retinitis, and uveitis.2,7,10,16,23,52, Linear scleroderma and Parry-Romberg syndrome 54,62,114,159,174 The condition is a form of lipodys- can both be associated with enophthalmos due to trophy. Histologically, Parry-Romberg syndrome re- orbital atrophy. The two diseases also share other sembles the sclerosis and perivascular leucocytic common features. However, the relationship infiltration seen in linear scleroderma, but in Parry- between linear scleroderma ‘‘en coup de sabre’’, Romberg syndrome there is preservation of elastic and Parry-Romberg syndrome remains controversial fibers.133 Treatment approaches include cosmetic and unclear.83,106,181 tissue augmentation of affected areas, consisting Linear scleroderma is a relatively rare disorder of transplantation of skin and subcutaneous characterized by localized, progressive fibrosis of fat,58,120,131,141 as well as symptomatic treatment of skin, subcutaneous fat, blood vessels, and muscles neurologic signs, anti-inflammatory regimens, and usually in the V1 dermatome (‘‘coup de sabre’’) stellate ganglion blocks.35,111,114 468 Surv Ophthalmol 52 (5) September--October 2007 HAMEDANI ET AL

after radiotherapy. Consequently, visual fields can be affected by direct compression of the optic nerve due to the primary orbital disease, radiotherapy, and enophthalmos.1 A characteristic facial appearance (hourglass facial deformity) can be induced following bilateral orbital irradiation. The radiologic findings of hypotelorism, enophthalmos, depressed temporal bones, atrophy of the temporalis muscles, narrow and deep orbits, and a depressed nasion can be seen early.60,186 Fig. 10. HIV infection-related lipodystrophy inducing Treatment of post-irradiation-related enophthal- bilateral enophthalmos. mos is quite challenging. Surgery in these tissues is unpredictable because the vascularity of the region G. AGE-RELATED FAT ATROPHY is uncertain. Acceptable results can be achieved using osteotomies, tissue expansion, repositioning, There is a natural involution of orbital fat with and bone grafting.15,84,91 age. This change also involves the other parts of the Radiotherapy for malignant tumors in adults face and temporal region. The age-related enoph- (e.g., lymphomas or metastasis) could be responsi- thalmos is bilaterally symmetric without any symp- ble for tissue scarring, fat atrophy, and resultant toms or pathologic findings. This fact is the basis for enophthalmos. lipofilling or liposculpting for facial rejuvenation. Although the position of the globe will not change, the increased fullness of the lids will improve the IV. Summary and Conclusion cosmetic aspect.34 The knowledge of the different etiologies of enophthalmos is important for further diagnostic H. HIV INFECTION--RELATED LIPODYSTROPHY steps and appropriate treatment. Although trauma HIV-infected patients often present with bilateral is the main cause of acquired enophthalmos, enophthalmos some years after the initiation of different other etiologies should be kept in mind: treatments (Fig. 10). Enophthalmos results from the 1) breast cancer metastasis because of the prognos- loss of subcutaneous fat in the face, mainly at the tic importance of early diagnosis, 2) orbital varix temporal areas and at the cheeks. These findings are because of the need to consider a diagnostic Valsalva induced mainly by nucleoside treatment and rapidly maneuver, and 3) chronic maxillary atelectasis lead to an enophthalmos due to the loss of orbital because of the possible reversibility in cases of early 112 fat. diagnosis.

I. RADIOTHERAPY The lateral effects of radiotherapy on the orbit V. Method of Literature Search depend mainly on the age of the patient. Thera- MEDLINE was used to search the literature from peutic radiation in childhood (mostly for retino- 1980 to 2006. Supplemental sources including blastoma or rhabdomyosarcoma) can efficiently Index Medicus and references contained in identi- treat facial or orbital malignancy. Nevertheless, it fied articles were used. The English abstracts of might also lead to visible disfigurement including foreign language articles were also included as well enophthalmos due to irradiation of developing as our personal reference libraries citing articles in structures that are in close proximity but not part French and German. Keywords searched were: of the intended target.116 These alterations explain enophthalmos, enophthalmia, enophthalmos and fractures, the effort to develop techniques for more accurate enophthalmos and implants, enophthalmos AND Horner, treatment of the tumoral region sparing the enophthalmos AND ptosis, pseudoenophthalmos, enoph- surrounding tissues, that is, with proton-radiation thalmos AND anophthalmos enophthalmos AND facial therapy.77 The incidence for significant late enoph- hemiatrophy, enophthalmos AND scleroderma enophthal- thalmos was found to be 28% by the Intergroup mos AND lipodystrophy, enophthalmos AND fibrosis, Rhabdomyosarcoma Study.137 Osseous hypoplasia enophthalmos AND orbital asymmetry, enophthalmos and atrophy of orbital content are at the origin of AND fat atrophy, enophthalmos AND metastasis enoph- these deformities. Therefore, younger children are thalmos AND breast cancer enophthalmos AND orbital more susceptible with the possibility of more severe varix, enophthalmos AND neurofibromatosis, enophthal- secondary skull deformations and enophthalmos mos AND chronic maxillary atelectasis, enophthalmos DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 469

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Soparkar CN, Patrinely JR, Cuaycong MJ, et al: The silent and Rothschild Foundation (Patrice More`re, Paris) for their sinus syndrome. A cause of spontaneous enophthalmos. contribution. The authors would also like to thank A Goldman, Ophthalmology 101:772--8, 1994 MD, (Boulder, CO) for his assistance in editing this paper. 165. Soparkar CN, Patrinely JR, Davidson JK: Silent sinus Reprint address: Mehrad Hamedani, MD, Jules Gonin Eye syndrome-new perspectives? Ophthalmology 111:414--5, Hospital, University of Lausanne, Avenue de France 15, Case author reply 415--6, 2004 postale 133 - 1000 Lausanne 7, Switzerland. DIAGNOSIS AND MANAGEMENT OF ENOPHTHALMOS 473

Outline c. Facial asymmetry

I. Diagnosis of enophthalmos II. Pathophysiology of enophthalmos A. Deﬁnition A. Enlargement of the orbital container B. Clinical presentation B. Reduction of the orbital content 1. Symptoms C. Contraction of the orbital content 2. Clinical examination III. Etiologies of enophthalmos and management C. Radiological imaging A. Post-traumatic enophthalmos D. Pseudoenophthalmos B. Chronic maxillary atelectasis (silent sinus 1. Globe syndrome) C. Recklinghausen disease a. Phthisis bulbi D. Orbital varix b. Microphthalmos, microcornea E. Breast cancer metastasis c. Refractive-anisometropia F. Linear scleroderma/ Parry Romberg syn- 2. Altered lid position drome (hemifacial atrophy) G. Age-related fat atrophy a. Horner syndrome H. HIV infection--related lipodystrophy b. Ptosis I. Radiotherapy 3. Structural lesions IV. Summary and conclusion a. Post-enucleation socket syndrome V. Method of literature search (PESS) b. Contralateral proptosis/exophthalmos